Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)

Aim Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter stud...

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Veröffentlicht in:Clinical rheumatology 2023-10, Vol.42 (10), p.2855-2860
Hauptverfasser: Bağlan, Esra, Kızıldağ, Zehra, Çağlayan, Şengül, Çakmak, Figen, Yener, Gülçin Otar, Özdel, Semanur, Öztürk, Kübra, Makay, Balahan, Çakan, Mustafa, Ayaz, Nuray Aktay, Sözeri, Betül, Ünsal, Şevket Erbil, Bülbül, Mehmet
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container_end_page 2860
container_issue 10
container_start_page 2855
container_title Clinical rheumatology
container_volume 42
creator Bağlan, Esra
Kızıldağ, Zehra
Çağlayan, Şengül
Çakmak, Figen
Yener, Gülçin Otar
Özdel, Semanur
Öztürk, Kübra
Makay, Balahan
Çakan, Mustafa
Ayaz, Nuray Aktay
Sözeri, Betül
Ünsal, Şevket Erbil
Bülbül, Mehmet
description Aim Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. Materials and methods The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. Results The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% ( n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. Conclusion In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times.
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It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. Materials and methods The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. Results The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% ( n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. Conclusion In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times.</description><identifier>ISSN: 0770-3198</identifier><identifier>EISSN: 1434-9949</identifier><identifier>DOI: 10.1007/s10067-023-06677-7</identifier><identifier>PMID: 37378874</identifier><language>eng</language><publisher>Cham: Springer International Publishing</publisher><subject>antibodies ; demographic statistics ; Demography ; Fascia ; females ; immunosuppression ; Immunosuppressive agents ; Medicine ; Medicine &amp; Public Health ; Methotrexate ; muscles ; Original Article ; Patients ; Pediatrics ; relapse ; Remission ; retrospective studies ; Rheumatology ; risk ; Risk factors ; Scleroderma ; Skin diseases ; therapeutics</subject><ispartof>Clinical rheumatology, 2023-10, Vol.42 (10), p.2855-2860</ispartof><rights>The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR) 2023. 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It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. Materials and methods The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. Results The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% ( n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. Conclusion In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. 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It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment. Materials and methods The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups. Results The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% ( n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%. Conclusion In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients. Key points • Most of the pediatric morphea patients respond well to MTX. • Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients. • Presence of extracutaneous findings in patients increased relapse rate 5.7 times.</abstract><cop>Cham</cop><pub>Springer International Publishing</pub><pmid>37378874</pmid><doi>10.1007/s10067-023-06677-7</doi><tpages>6</tpages><orcidid>https://orcid.org/0000-0002-7938-2560</orcidid><orcidid>https://orcid.org/0000-0002-1034-6406</orcidid><orcidid>https://orcid.org/0000-0001-5602-4595</orcidid><orcidid>https://orcid.org/0000-0002-8800-0800</orcidid><orcidid>https://orcid.org/0000-0003-0466-0228</orcidid><orcidid>https://orcid.org/0000-0002-5079-5644</orcidid><orcidid>https://orcid.org/0000-0003-3014-5692</orcidid><orcidid>https://orcid.org/0000-0003-3594-7387</orcidid><orcidid>https://orcid.org/0000-0002-1667-2480</orcidid><orcidid>https://orcid.org/0000-0001-9007-9653</orcidid><orcidid>https://orcid.org/0000-0003-2575-6309</orcidid><orcidid>https://orcid.org/0000-0001-6193-0402</orcidid><orcidid>https://orcid.org/0000-0001-5637-8553</orcidid></addata></record>
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issn 0770-3198
1434-9949
language eng
recordid cdi_proquest_miscellaneous_3153199326
source SpringerLink (Online service)
subjects antibodies
demographic statistics
Demography
Fascia
females
immunosuppression
Immunosuppressive agents
Medicine
Medicine & Public Health
Methotrexate
muscles
Original Article
Patients
Pediatrics
relapse
Remission
retrospective studies
Rheumatology
risk
Risk factors
Scleroderma
Skin diseases
therapeutics
title Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)
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